Causa muy rara de pubertad precoz periférica en una niña: tumor de cordones sexuales con túbulos anulares / A very rare cause of peripheral precocious puberty in a girl: ovarian sex cord tumor with annular tubules

El tumor de los cordones sexuales con túbulos anulares es una neoplasia del estroma gonadal muy infrecuente. Representa el 0,05-0,6% de todos los tumores ováricos, según series. Se presenta un caso especialmente inusual, en una niña de 6 años, detectado a raíz de una pubertad precoz periférica isosexual. Su interés radica en que no se halló ninguna masa anexa al ovario, sino únicamente una asimetría gonadal, sin signos radiológicos de malignidad. Se realizó una salpingo-ooforectomía unilateral con linfadenectomía pélvica y paraaórtica ipsilateral, por vía laparoscópica, tras confirmarse la presencia de células tumorales en la biopsia intraoperatoria. La evolución posterior de la paciente fue favorable.

Sex cord tumor with annular tubules is an extremely uncommon gonadal stromal neoplasm. It represents 0.05-0.6% of all ovarian tumors, according to series. An unusual case is presented in a 6-year-old girl, detected as a result of an isosexual peripheral precocious puberty. The highlight of this case is that no mass attached to the ovary was found, but only a gonadal asymmetry without radiological signs of malignancy. After confirming the presence of tumoral cells by intraoperative biopsy, unilateral salpingo-oophorectomy with ipsilateral para-aortic and pelvic lymphadenectomy was performed. Afterwards, the evolution of the patient was favorable.

Hyperandrogenism produced by ovarian tumors in women at different life stages / Hyperandrogenism produced by ovarian tumors in women at different life stages.

OBJECTIVE: to evaluate the different presentations of hyperandrogenism produced by ovarian tumors in women at different life stages DESIGN: case report. SETTING: academic institutions. PATIENT(S): 3 patients at different life stages, with increased androgen levels. INTERVENTION(S): review of hospital records. MAIN OUTCOME MEASURE(S): clinical and biochemical features, treatment and follow-up. RESULT(S): a 10 year-old girl with Leydig cell tumor presented with hyperandrogenemia, virilization and changes in social behavior. Another patient, at reproductive age, with a tumor>10 cm, presented with signs of virilization and abdominal mass, whose pathologic analysis disclosed a carcinoid tumor of the ovary associated with stromal hyperplasia. The third patient was a postmenopausal woman with severe alopecia, who presented a steroid cell tumor, rare at that age. CONCLUSION(S): the evaluation of women with signs and symptoms of virilization should include a detailed clinical record, thorough physical examination and an appropriate hormonal dosage, especially when images are inconclusive.

Tumor estromal esclerosante de ovário associado à síndrome de Meigs e gestação: relato de caso / Sclerosing stromal tumor of the ovary associated with a Meigs' syndrome and pregnancy: a case report

O tumor estromal esclerosante de ovário é uma neoplasia benigna extremamente rara, mais frequente em mulheres jovens e sem sintomas específicos na maioria dos casos. Menos de 150 casos foram descritos, dos quais 8 diagnosticados durante a gestação. Neste relato, documentamos a associação entre tumor estromal esclerosante de ovário, síndrome de Meigs e elevação dos níveis de CA-125 em gravidez a termo.

The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.

Carcinoma endometroide con áreas semejantes a tumor estromal de cordones sexuales / Endometrioid carcinoma with similar areas of sex cord stromal tumor

Conocido antiguamente como el "asesino silencioso", el cáncer de ovario muestra un espectro de síntomas inespecíficos que, una vez reconocidos, facilitan un diagnóstico temprano y prolongan la supervivencia de la paciente. Se presenta el caso de una paciente de 57 años referida de consulta privada por sangrado post menopaúsico con US transvaginal que revela datos de malignidad ovárica, laboratorios de rutina y marcadores tumorales normales...

Refractory Hypertension and Isosexual Pseudoprecocious Puberty Associated with Renin-Secreting Ovarian Steroid Cell Tumor in a Girl

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.

Peutz-Jeghers Syndrome with Multiple Genital Tract Tumors and Breast Cancer: A Case Report with a Review of Literatures

We report here on the multiple genital tract neoplasms in a 41-yr-old Korean woman with Peutz-Jeghers Syndrome (PJS). The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer. Pelvic ultrasound showed a multilocular cyst at the right adnexal region, diagnosed as bilateral ovarian mucinous borderline tumors. An ovarian sex cord tumor with annular tubules was incidentally diagnosed together with a minimal deviation adenocarcinoma of the uterine cervix and mucinous metaplasia of both the Fallopian tubal mucosa and the endometrium. Although the cases of multiple genital tract tumors with PJS has rarely been reported, the present case appears to be the first in Korea in which the PJS syndrome was complicated by multiple genital tract tumors and infiltrating carcinoma of the breast. The clinical significance of the multiple genital tract tumors and breast cancer associated with PJS is reviewed.

Testicular tumour with features of sex cord tumor with annular tubules associated with cryptorchidism and infertility--a case report.

Sex cord tumor with annular tubules (SCTAT) is a rare tumor seen in the ovary usually associated with Peutz-Jeghers syndrome. Testicular SCTAT are more infrequent and only four such cases have been reported in the literature. A 28-year-male presented with infertiliy. He had a history of orchiopexy 10 years back for right-sided inguinal testes. A right testicular biopsy done to investigate the cause of infertility revealed testicular atrophy with a focus suspicious of SCTAT. The right-sided orchiectomy revealed two firm nodules of 0.5 and 0.2 cms of SCTAT and sertoli cell nodule (SCN) respectively on microscopy. He did not have any features of Peutz-Jeghers or any other dysgenetic syndrome. He is asymptomatic after follow up of 26 months. This is the fifth case of testicular SCTAT and the first with SCN.